[Myelodysplastic syndromes. Hematologic phenotypes, cytogenetic expression and clinical course in 133 cases (1979-1989)].
One hundred and thirty-three cases of myelodysplastic syndromes studied during the last ten years were revised. Of them, 79 were males and 54 females, and their ages ranged between 15 and 91 years (median, 69 years). Five patients (3.7%) had secondary myelodysplasias. The haematological phenotype (FAB) of the cases was: RA, 41.3%; SRA, 24%; RAEB, 18%; RAEBT, 3.7%; CMML, 8.3%. Leucopenia/thrombocytopenia without initial anaemia was present in 4.5% of the cases. Abnormal karyotype was found in 54 patients (40.6%), MIKA in 41 cases and MAKA in 13 cases. The cytogenetic anomalies most commonly found were +8, 5q-, -7, 11q- and 13q-. Cytogenetic abnormalities were commonest amongst the RAEB (50%), and least frequent in CMML (18.2%). Thirty-one patients evolved into acute leukaemia (29 ANLL and 2 ALL). Such blastic changes were more frequent in RAEB (62.5%) and rarest in SRA (9.4%), and they appeared mostly in patients with complex karyotype (MAKA) (53.8%) as compared with those who had normal karyotype (17.7%). Short-lasting complete remission was achieved by 40% of the patients treated with conventional chemotherapy. The survival of the group as a whole (median 30 months) varied in accordance with the haematological phenotype: SRA, 81 months; RA, 65 months; CMML, 13 months; RAEB +/- T, 8 months. The finding of a MAKA karyotype significantly shortened the survival (4 months) with regard to MIKA (44 months) or normal karyotype (39 months). The following median survivals were attained after patients' staging (Bournemouth's criteria): stage A, 84 months; stage B, 22 months, and stage C, 5 months.(ABSTRACT TRUNCATED AT 250 WORDS)
Sánchez Fayos
J
J
Servicio de Hematología y Genética, Fundación Jiménez Díaz, Facultad de Medicina, Universidad Autónoma, Madrid.
Pérez Rus
G
G
Benitez
J
J
Outeiriño
J
J
Prieto
E
E
Calabuig
T
T
Pérez Sáenz
M A
MA
Rodríguez
C
C
Román
A
A
Sánchez Castaña
A
A
D000206:Actuarial Analysis; D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002869:Chromosome Aberrations; D015331:Cohort Studies; D005260:Female; D006801:Humans; D007938:Leukemia / Q000453:epidemiology / Q000209:etiology; D008297:Male; D008875:Middle Aged; D009190:Myelodysplastic Syndromes / Q000145:classification / Q000453:epidemiology / Q000235:genetics / Q000628:therapy; D010641:Phenotype; D011289:Preleukemia / Q000453:epidemiology; D012189:Retrospective Studies; D013030:Spain / Q000453:epidemiology; D015996:Survival Rate
Síndromes mielodisplásticos. Fenotipos hematológicos, expresión citogenética y patrones evolutivos de 133 casos (1979-1989).